8.
Henry DeMots, MD
Professor of Medicine (OHSU)
Medical Director, University Medical Group
demotsh@ohsu.edu
Clinical Focus: Dr. DeMots’ special interests are in heart failure and coronary artery disease. He is
widely recognized for his teaching contributions in the field of inpatient cardiology. He received his medical
degree in 1966 from Northwestern University in Chicago, and completed his residency and fellowship at
OHSU. Dr. DeMots has been board certified in Internal Medicine and Cardiology since 1966.
George D. Giraud, MD, PhD
Associate Professor of Medicine (VAMC)
giraudg@ohsu.edu, george.giraud@va.gov
Clinical Focus: Dr. Giraud’s clinical interest is in cardiovascular imaging.
Research Interest: Dr. Giraud is working closely with Kent Thornburg in studies on fetal cardiac
growth, regulation and development. A specific area of focus is the effect of maternal-fetal
environment on contractile physiology and the biochemical indicators involved.
Susan Grauer, MD
Assistant Professor of Medicine (VAMC)
grauers@ohsu.edu, susan.grauer@va.gov
Clinical Focus: Dr. Grauer is a clinician educator who practices general cardiology with a focus
on echocardiography and with special interest in valvular heart disease and pericardial disease.

27.
Joseph B. Weiss, MD, PhD
Assistant Professor of Molecular Medicine & Cardiology
(OHSU)
weissjo@ohsu.edu
Clinical Focus: Dr. Weiss practices general cardiology with a specific focus on adult congenital
heart disease.
Research Interests: The Weiss lab studies heart and smooth muscle development in
Drosophila and mice. This accomplished research group takes advantage of the evolutionary
conservation of developmental mechanisms and the power of developmental genetics in
Drosophila to advance our understanding of mammalian organogenesis. One striking example of
conserved developmental mechanisms is the homeodomain protein Tinman. Homozygous
Drosophilia tinman mutant embryos have neither heart nor smooth muscle. Heterozygous human
mutants in the Tinman homologue Nkx2.5 evince a broad spectrum of congenital heart lesions.
Starting with this evolutionarily conserved transcriptional regulator of heart and smooth muscle
development, we have established a method to identify genes that are direct targets of Tinman
regulation.

28.
Zhengfeng Zhou, MD, PhD
Assistant Professor of Medicine (OHSU)
Assistant Professor of Physiology and Pharmacology
zhouzh@ohsu.edu
Research Interests: The research in Dr. Zhou’s lab is to study molecular mechanisms of
genetic diseases involving cardiac arrhythmias. They have concentrated their studies on two
congenital diseases, long QT syndrome (LQTS) and arrhythmogenic right ventricular dysplasia
(ARVD). LQTS is a disease associated with prolonged cardiac action potential duration and
prolonged QT intervals on ECG, which can lead to ventricular arrhythmia and sudden death. One of
the major forms of LQTS (LQT2) is caused by mutations in the human ether-a-go-go-related gene
(hERG) that encodes a voltage-gated potassium channel in the heart. To date, more than 300 hERG
mutations have been identified in patients with LQTS. Current studies are investigating the
mechanisms of defective protein trafficking of LQT2 mutant channels, and determining how LQT2
mutations lead to defects in splicing and stability of mutant mRNA. ARVD is an inherited disorder
characterized by prominent arrhythmias and the replacement of muscle in the heart by fatty tissue.
Eight disease-causing genes have been identified in autosomal dominant and recessive forms of
ARVD. Most of these genes encode desmosomal proteins including plakoglobin, desmoplakin,
plakophilin-2, desmoglein-2, and desmocollin-2. A prominent feature of ARVD mutations is that
more than 60% of these mutations are nonsense or frameshift mutations that introduce premature
termination codons. The goal of this project is to determine the role of the nonsense-mediated
mRNA decay (NMD) pathway in the pathogenesis of ARVD mutations. Understanding how these
genetic defects lead to the disease at molecular levels will provide important information directed
toward the development of therapeutic strategies for patients with LQTS and ARVD.
Sample Publications
Anderson CL, Delisle BP, Anson BD, Kilby JA, Will ML, Tester DJ, Gong Q, Zhou Z, Ackerman MJ
and January CT. Most LQT2 mutations reduce Kv11.1 (hERG) current by a class 2 (trafficking-
deficient) mechanism. Circulation 113:365-373, 2006.
Gong Q, Jones MA and Zhou Z. Mechanisms of pharmacological rescue of trafficking-defective
hERG mutant channels in human long QT syndrome. J. Biol. Chem 280:4069-4074, 2006.
Gong Q, Zhang L, Vincent GM, Horne BD and Zhou Z. Nonsense mutations in hERG cause a
decrease in mutant mRNA transcripts by nonsense-mediated mRNA decay in human long QT
syndrome. Circulation 116:17-24, 2007.
Bhuiyan ZA, Momenah TS, Gong Q, Amin AS, Ghamdi SA, Carvalho JS, Homfray T, Mannens MMAM,
Zhou Z, Wilde AAM. Recurrent intrauterine fetal loss due to near absence of HERG: Clinical and
functional characterization of a homozygous nonsense HERG mutation. Heart Rhythm 5:553-
561, 2008.
Gong Q, Zhang L, Moss AJ, Vincent GM, Ackerman MJ, Robinson JC, Jones MA, Tester DJ and Zhou
Z. A splice site mutation in hERG leads to cryptic splicing in human long QT syndrome. J. Mol.
Cell. Cardiol. 44:502-509, 2008.